Evaluation of mitochondrial bioenergetics, dynamics, endoplasmic reticulum-mitochondria crosstalk, and reactive oxygen species in fibroblasts from patients with complex I deficiency

Interaction of Mitochondria with the Endoplasmic Reticulum and Plasma Membrane in Calcium Homeostasis, Lipid Trafficking and Mitochondrial Structure

(2017) Jędrzej Szymański et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Severe defect in mitochondrial complex I assembly with mitochondrial DNA deletions in ACAD9 -deficient mild myopathy

(2017) Konstantina Fragaki et al.   MUSCLE & NERVE

Biallelic Mutations in TMEM126B Cause Severe Complex I Deficiency with a Variable Clinical Phenotype

(2016) Charlotte L. Alston et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mitochondrial complex I-linked disease

(2016) Richard J. Rodenburg   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Structure and function of mitochondrial complex I

(2016) Christophe Wirth et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Mitochondrial disorders in children: toward development of small-molecule treatment strategies

(2016) W. J. Koopman et al.   EMBO Molecular Medicine

Neonatal multiorgan failure due to ACAD9 mutation and complex I deficiency with mitochondrial hyperplasia in liver, cardiac myocytes, skeletal muscle, and renal tubules

(2016) Nancy Leslie et al.   HUMAN PATHOLOGY

Mitochondria-associated endoplasmic reticulum membranes allow adaptation of mitochondrial metabolism to glucose availability in the liver

(2016) Pierre Theurey et al.   Journal of Molecular Cell Biology

A Mitochondrial-Targeted Nitroxide Is a Potent Inhibitor of Ferroptosis

(2016) Tanja Krainz et al.   ACS Central Science

Redefining the roles of mitochondrial DNA-encoded subunits in respiratory Complex I assembly

(2015) Rasika Vartak et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Complex I assembly function and fatty acid oxidation enzyme activity of ACAD9 both contribute to disease severity in ACAD9 deficiency

(2015) Manuel Schiff et al.   HUMAN MOLECULAR GENETICS

Titanium Dioxide Nanoparticles Induce Endoplasmic Reticulum Stress-Mediated Autophagic Cell Death via Mitochondria-Associated Endoplasmic Reticulum Membrane Disruption in Normal Lung Cells

(2015) Kyeong-Nam Yu et al.   PLoS One

Amelioration of Radiation-Induced Oral Cavity Mucositis and Distant Bone Marrow Suppression in Fanconi AnemiaFancd2–/–(FVB/N) Mice by Intraoral GS-Nitroxide JP4-039

(2014) Hebist Berhane et al.   RADIATION RESEARCH

The molecular hug between the ER and the mitochondria

(2013) Benoît Kornmann   CURRENT OPINION IN CELL BIOLOGY

ACAD9, a complex I assembly factor with a moonlighting function in fatty acid oxidation deficiencies

(2013) Jessica Nouws et al.   HUMAN MOLECULAR GENETICS

TIMMDC1/C3orf1 Functions as a Membrane-Embedded Mitochondrial Complex I Assembly Factor through Association with the MCIA Complex

(2013) Virginia Guarani et al.   MOLECULAR AND CELLULAR BIOLOGY

Complex I deficiency: clinical features, biochemistry and molecular genetics

(2012) Elisa Fassone et al.   JOURNAL OF MEDICAL GENETICS

Endoplasmic reticulum–mitochondria contacts: function of the junction

(2012) Ashley A. Rowland et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Superoxide-lowering therapy with TEMPOL reverses arterial dysfunction with aging in mice

(2011) Bradley S. Fleenor et al.   AGING CELL

Two Strategies for the Development of Mitochondrion-Targeted Small Molecule Radiation Damage Mitigators

(2011) Jean-Claude M. Rwigema et al.   INTERNATIONAL JOURNAL OF RADIATION ONCOLOGY BIOLOGY PHYSICS

Large-Scale Asymmetric Synthesis of the Bioprotective Agent JP4-039 and Analogs

(2011) Marie-Céline Frantz et al.   ORGANIC LETTERS

Mitochondrial Structure and Function Are Disrupted by Standard Isolation Methods

(2011) Martin Picard et al.   PLoS One

GS-Nitroxide (JP4-039)-Mediated Radioprotection of Human Fanconi Anemia Cell Lines

(2011) Mark E. Bernard et al.   RADIATION RESEARCH

Mitochondrial bioenergetics and dynamics interplay in complex I-deficient fibroblasts

(2010) M. Morán et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Acyl-CoA Dehydrogenase 9 Is Required for the Biogenesis of Oxidative Phosphorylation Complex I

(2010) Jessica Nouws et al.   Cell Metabolism

Evidence for Physical Association of Mitochondrial Fatty Acid Oxidation and Oxidative Phosphorylation Complexes

(2010) Yudong Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Coupling mitochondrial dysfunction to endoplasmic reticulum stress response: A molecular mechanism leading to hepatic insulin resistance

(2008) J LIM et al.   CELLULAR SIGNALLING

Modulation of mitochondrial morphology by bioenergetics defects in primary human fibroblasts

(2008) O. Guillery et al.   NEUROMUSCULAR DISORDERS